Multi-vessel coronary artery aneurysms in a patient with Parry Romberg syndrome: a case report.

Background: Coronary artery aneurysms (CAAs) are a very rare finding on coronary angiograms with multiple known aetiologies. Parry Romberg syndrome (PRS) is also a very rare disease, and the underlying aetiology remains unknown. We present a rare case of CAAs in a patient with PRS, and discuss possible implications regarding the primary pathophysiological cause for both of these diseases.

Case Summary: A 48-year-old woman with a history of PRS presented with atypical and non-exertional chest pain. Initial evaluation demonstrated a rising troponin without associated electrocardiogram changes, and as such she was taken for left heart catheterization. Left heart catheterization demonstrated diffuse aneurysmal and ectatic disease of multiple coronary arteries. Further evaluation with magnetic resonance angiogram and autoantibody panel did not demonstrate other vascular anomalies or rheumatologic disease, respectively. She was treated with dual anti-platelet therapy and statin, and at 1 year follow-up, she had resolution of her symptoms.

Discussion: It has been postulated that the underlying mechanism causing CAA is intravascular inflammation. Parry Romberg syndrome is theorized to be a neurovasculopathy, as evidenced by cases of associated intracranial aneurysms. Intravascular inflammation may play a key pathological role in CAA, and an association between CAA and PRS may exist.